title: Acute encephalopathy with unilateral cortical-subcortical lesions in two unrelated kindreds treated with glucocorticoids prenatally for congenital adrenal hyperplasia due to 21-hydroxylase deficiency: established facts and novel insight.
abstract: BACKGROUND
  Prenatal glucocorticoid (GC) treatment of the female fetus with 21-hydroxylase deficiency (21-OHD) may prevent genital virilization and androgen effects on the brain, but prenatal GC therapy is controversial because of possible adverse effects on fetal programming, the cardiovascular system and the brain.
  
  
  METHODS
  We report 2 patients with congenital adrenal hyperplasia (CAH) due to 21-OHD who were treated prenatally with dexamethasone, suffered from an acute encephalopathy and showed focal and multifocal cortical and subcortical diffusion restrictions in early MRI and signs of permanent alterations in the follow-up neuroimaging studies. Both patients recovered from the acute episode. Whereas the first patient recovered without neurological sequelae the second patient showed hemianopsia and spastic hemiplegia in the neurological follow-up examination.
  
  
  CONCLUSIONS
  These are 2 children with CAH, both treated prenatally with high doses of dexamethasone to prevent virilization. The question arises whether prenatal high-dose GC treatment in patients with CAH might represent a risk factor for brain lesions in later life. Adverse effects/events should be reported systematically in patients undergoing prenatal GC treatment and long-term follow-up studies involving risk factors for cerebrovascular disease should be performed.
authors.affiliation: Department of Pediatrics, University Children's Hospital, University Hospital Inselspital, Bern, Switzerland.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Grunt
authors.forename: Maja
authors.initials: M
authors.lastname: Steinlin
authors.forename: Christian
authors.initials: C
authors.lastname: Weisstanner
authors.forename: Martin
authors.initials: M
authors.lastname: Schöning
authors.forename: Primus E
authors.initials: PE
authors.lastname: Mullis
authors.forename: Christa E
authors.initials: CE
authors.lastname: Flück
mesh_terms: D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D003907:Dexamethasone / Q000009:adverse effects*; D005260:Female; D005333:Fetus / Q000187:drug effects*; D006801:Humans; D007223:Infant; D008297:Male; D020258:Neurotoxicity Syndromes / Q000209:etiology*; D011247:Pregnancy; D011295:Prenatal Care; D011297:Prenatal Exposure Delayed Effects; D014770:Virilism / Q000517:prevention & control
chemical_list: D003907:Dexamethasone